Sickle Cell Anemia 2007

Sickle Cell Anemia

Definition 

Sickle cell anemia is a disease passed down through families in which red blood cells form an abnormal crescent shape.

Causes/Etiology 

Sickle Cell Anemia is an inherited disorder in which a child inherits one abnormal gene from each parent. This is known as autosomal recessive inheritance.

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abnormal genes produce an abnormal type of blood hemoglobin called hemoglobin S.  RBC containing HbS loses its round, pliable, biconcave disk shape and becomes deformed, rigid and sickled-shaped  Sickle-shaped cells deliver↓O2 to the body's tissues. They also can clog more easily in small blood vessels, and break into pieces that disrupt blood flow, the patient may have pain, swelling and fever  If the RBC is again exposed to adequate amount of oxygen before the membrane becomes too rigid, it can revert to a normal shape  Cold can aggravate the sickling process

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Risk Factor 

NON MODIFIABLE

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Hereditary 2. Race Sickle cell anemia is most common in people of African, Indian, Middle Eastern, or Mediterranean descent. Nevertheless, it can occur in all peoples. 1.

Signs and Symptoms Signs and symptoms of sickle cell anemia usually show up after an infant is 4 months old 

Includes: Anemia RBCs usually live for about 120 days before they die and need to be replaced. However, sickle cells die after only 10 to 20 days.

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Without enough red blood cells in circulation, your body can't get the oxygen it needs to feel energized.

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 Delayed growth RBCs provide your body with the oxygen and nutrients

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you need for growth Shortage of healthy red blood cells can slow growth in

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infants and children and delay puberty in teenagers

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 Jaundice 

It is a yellowing of the skin and eyes that occurs because of liver damage or dysfunction

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Sickle cell anemia have some degree of jaundice

 Hand-foot 

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syndrome Swollen hands and feet may be the first signs of sickle cell anemia in babies. The swelling is caused by sickleshaped red blood cells blocking blood flow out of their hands and feet.  Frequent infection Sickle cells can damage your spleen, an organ that fights infection. This may make you more vulnerable to infections.

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1year old and up Vision Problem 

Tiny blood vessels that supply your eyes may become plugged with sickle cells. This can damage the retina (portion of the eye that processes visual images).

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Episodes of pain (Sickle Cell Crisis)

Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to your chest, abdomen and joints. Pain can also occur in your bones.

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The pain may vary in intensity and can last for a few hours to a few weeks.

Diagnostic Test Test

Hemoglobin electrophoresis

Definition

Purpose

Hemoglobin electrophoresis is a test that measures the different types of the oxygencarrying substance (hemoglobin) in the blood.

You may have this test if your health care provider suspects that you have a disorder caused by abnormal forms of hemoglobin

Normal result

Abnormality with condition

No HbS

Presence of HbS

Sickle Cell  Test

A sickle This test is cell test done to tell looks for if you have the presenceabnormal or absence hemoglobin of abnormal that causes hemoglobin sickle cell in the blood disease and that causes sickle cell sickle cell trait. anemia.

Negative

Sickle Cell  Trait Sickle Cell  Anemia

Complete Blood Count (CBC)

-It measures the # of RbCs, # of WBCs, total amount It is a of Hb in the screening test blood, % of used to the blood diagnose and composed of manage RBCs(hemato numerous crit) diseases. -provides specific information the size and hemoglobin content of individual red blood cells.

RBC: Male: 4.7 to 6.1million cells/mcL

decrease (indicates

Female: 4.2 to 5.4 million cells/mcL

WBC:4,500 to 10,000 cells/mcL

anemia)

Hgb: Male: 13.8 to 17.2 gm/dL

Chronic

15.1 gm/dL

neutrophilia

Female: 12.1 to

often present

is

Platelets: decrease 1500,000400,000/µL 5-10g/dL often increase

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Nursing Management  Provide

information about what the client may feel  Encourage questions and dialogue about fears and apprehensions  Inform the client of the time period before the results will be available

Management 

Medical  Patients

with sickle cell disease need ongoing treatment, even when they are not having a painful crisis. 

Classification: Analgesic

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Action: It is used for the relief of fever as well as aches and pains associated with many conditions.

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Hydroxyurea

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Classification: Antimetabolite

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Action: inhibits production of Hb F, which inhibits sickling

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Indication: to reduce frequency of painful crises and need for blood transfusions in adult patient with Sickle Cell Anemia with moderate to severe painful

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crises

Generic (Brand) names: Hydroxyurea (Droxia, Hydrea)

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recurrent

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3.

Blood Transfusion In a red blood cell transfusion, red blood cells are

removed from a supply of donated blood. These donated cells are then given intravenously to a person with sickle cell anemia. 

Blood transfusions increase the number of normal red blood cells in circulation, helping to relieve anemia. In children with sickle cell anemia at high risk of stroke, regular blood transfusions can decrease their risk of stroke.

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4. Supplemental oxygen

Breathing supplemental oxygen through a breathing mask adds oxygen to your blood and helps you breathe easier. 

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Pharmacological-Highlights

1.Supplements of Folic Acid(Vitamin B) 2.Fluids

3.Heating pads

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Surgical  Bone 

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marrow transplant This procedure replaces bone marrow affected by sickle cell anemia with healthy bone marrow from a donor who doesn't have the disease. It can be a cure, but the procedure is risky, and it's difficult to find suitable donors. Bone marrow used for a transplant must come from a closely matched donor. This is usually a close family member who doesn't have sickle cell anemia. Bone marrow transplant requires a lengthy hospital stay. After the transplant, you'll need drugs to help prevent rejection of the donated marrow.

Nursing  Diag nosis Diagnosis: Acute pain related to tissue hypoxia Interventions:  -promote calm and relaxing environment to divert client’s awareness to pain  -divertional activities like watching television, use of imagery etc.  -perform warm compress on the site of pain to provide relief  -encourage families to maintain a normal life for the child with SC Anemia Outcome: Client will be able to relieve pain. 

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Diagnosis: Risk for infection related to inadequate secondary defense Interventions:  -promote childhood immunization program  -encourage adults to update immunizations to reduce the transmission of global infection  -emphasize necessity of taking antiviral/antibiotics  -review individual nutritional needs, appropriate exercise program and need for rest  -encourage frequent proper hand hygiene Outcome: Client will be able to prevent the spread of potentially infectious microorganism. 

Diagnosis: Impaired gas exchange related to oxygen carrying capacity of the blood Interventions:  -review oxygen-conserving techniques  -instruct in the use of relaxation, stress reduction techniques  -encourage appropriate activity and exercise to improve quality of life  -emphasize the importance of nutrition in improving stamina and reducing the work of breathing Outcome: Client will be able to have an adequate oxygenation of tissue. 

Prog nosis 

In the past, sickle cell patients often died from organ failure between ages 20 and 40. Thanks to a better understanding and management of the disease, today, patients can live into their 50s or beyond.

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Causes of death include organ failure and infection. Some people with the disease experience minor, brief, infrequent episodes. Others experience severe, long-term, frequent episodes with many complications.

Re se arch Red grape skin may help sickle cell sufferers(October 31, 2009)  "AUGUSTA, Ga. – An extract in red grape skin may be a new treatment for sickle cell disease, Medical College of Georgia researchers say. Study Using Viagra Ingredient to Treat Sickle Cell Cut Short (July 28, 2009, 2:03 PM) 

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Viagra treats erectile dysfuntion by widening arteries. Viagra’s active ingredient also has been approved, under the name Revatio, to treat pulmonary hypertension, the condition raising blood pressure in the lungs. And that’s why researchers were studying whether Revatio might also work in patients with sickle-cell disease, a genetic blood disorder. www.newstin.com.uk

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Re fe re nce s Brunner and Suddarath’s “Textbook of Med-

Surgical Nursing 11th Edition”  Kozier ans Erb’s Fundamentals of Nursing  Taber’s Cyclopedia Medical Dictionary, F.A. Davis  Disease and disorders Nursing Therapeutic Manual  Http://en.wikipedia.org/wiki/Sickle_Cell_Disease  http://www.nlm.nih.gov/medlineplus/ency/article/ 000527.htm  http://www.mayoclinic.com/health/sickle-cellanemia/DS00324  Nurses’s Pocket Guide 11th Edition